The Immunocompromised Patient Flashcards

1
Q

What is an immunocompromised host?

A

Immunocompromised host – state in which the system Is unable to respond appropriately and effectively to infectious microorganisms. This is due to a defect in one or more components of the immune system.

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2
Q

What is immunodeficiency?

A

Immunodeficiency is caused by a defect in one or more components of the immune system such as those in this picture.

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3
Q

What is PID (primary immunodeficiency)

A

PID – occurs due to congenital disorder – from an intrinsic gene defect resulting in a missing protein, missing cell and non-functional components. – can be autosomal of x-linked.

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4
Q

What is SID (secondary immunodeficiency)

A

Secondary immunodeficiency – this is acquired – due to an underling disease/treatment leading to a reduction in production/function of the immune components or an increase in the loss or catabolism of immune components.

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5
Q

When should immunodeficiency be suspected?

A

Severe – life threatening
Persistent – persists even when using standard treatment
Unusual – site and type of organism (opportunistic)
Recurrent – keeps coming back after treatment

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6
Q

Other than infections what else does PID make you susceptible to?

A

Not only does PID make you susceptible to infections but also to cancer as the immune system plays an important role in preventing cancerous cells from surviving.

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7
Q

How does the age Immunodeficiencies present at give insight to the type of deficiency they have?

A

Onset < 6 months highly suggests a T-cell or phagocytes defect (not antibody as currently receiving antibodies from mother in breast milk)
Onset between 6months-5 years often suggests a B cell/antibody or phagocyte defect
Onset > 5 years old and later in life usually suggests a B cell/antibody/complement r secondary immunodeficiency.

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8
Q

What type of deficiency are most PIDs and give some examples

A
The majority (65%) of PIDs are antibody deficiencies.
• X linked agammaglobulinaemia or Bruton’s disease – cannot produce antibodies due to a defect in B cell maturation – X linked condition
• Common variable immunodeficiency (CVID) – very low level of IgG, IgM and IgA
• Selective IgA deficiency – mostly asymptomatic
• IgG subclass deficiency – mostly asymptomatic
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9
Q

What is combined T and B cell deficiency?

A

• Severe combined immunodeficiency – SCID – inhibition of T cell development – called combined because if T cells are inhibited then so will B cells.

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10
Q

What Phagocytic defects are there?

A
  • Chronic granulomatous disease – phagocyte can’t produce reactive oxygen species so cannot do a respiratory burst
  • Severe congenital neutropenia
  • Cyclic neutropenia
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11
Q

What is Wiskott-Aldrich syndrome?

A

Wiskott-Aldrich syndrome – x linked condition characterised by eczema low platelet count (thrombocytopenia) and immune deficiency caused by decreased antibody production and inability of T cells to become polarised making it combined.

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12
Q

What is DiGeorge syndrome?

A

DiGeorge syndrome – autosomal dominant caused by a deletion of part of chromosome 22 causing a range of congenital malformations including immunodeficiency

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13
Q

What is Hyper IgE syndrome?

A

Hyper IgE syndrome – abnormal neutrophil chemotaxis due to decreased production of interferon gamma by T lymphocytes

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14
Q

What is Ataxia-telangiectasia

A

Ataxia-telangiectasia – autosomal recessive with many symptoms but the immunodeficiency side of things is caused by low antibody production and low Lymphocytes, particularly T cells

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15
Q

How does Chronic granulomatous disease commonly present?

A

Skin infections
Pulmonary Aspergillosis – Halo signs in HRCT scan from nodules in the lung – chronic fungal infection of the lungs. This is from opportunistic infections from Aspergillus species (fungus).

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16
Q

How are PID patients commonly managed?

A

Supportive treatment – infection prevention (prophylaxis), treat infections promptly and aggressively (passive immunisation), Nutritional support (vitamin A/D), Use UV-irradiated Cytomegalovirus negative blood products only and avoid live attenuated vaccines in patients with severe PIDs (SCID).

Specific treatment – regular immunoglobulin therapy (IV IG or SC IG) and SCID – hematopoietic stem cell therapy (Haematopoietic Stem Cell Transplant, 90% success)

Comorbidities – autoimmunity and malignancies, organ damaged (lung functional assessment) and avoid non-essential exposure to radiation.

17
Q

What is immunoglobulin replacement therapy?

A

Immunoglobulin Replacement Therapy
Goal – serum IgG > 8g/l – lifelong treatment
Different formulations – IV IG, SC IG in young patients
Conditions – CVID, XLA (Bruton’s disease) and hyper-IgM syndrome

18
Q

What causes SID?

A

Decreased production of immune component due to – malnutrition, infection (HIV), liver diseases and lymphoproliferative diseases.

19
Q

Why is the spleen so important?

A

Why is the spleen so important?
• Blood borne pathogens – encapsulate bacteria
• Antibody production – acute response IgM production and long term protection in IgG production
• Splenic macrophages – removal of opsonised microbes and removal of immune complexes.

20
Q

What does being Asplenic/Splenectomised mean for a patient?

A

Presentation – increases susceptibility to encapsulated bacteria (haemophilus Influenzae, strep pneumonia and Neisseria meningitidis. This is because these bacteria need to be opsonised and this usually happens from antibodies produced by the spleen.
OPSI (overwhelming post-splenectomy infection) – sepsis and meningitis (1-2% risk of death over 15 years)

21
Q

How do we manage Aplenic patients?

A

Management – penicillin prophylaxis (lifelong)
Immunisation against encapsulated bacteria
Medical alert bracelet or card

22
Q

Why might someone have had their speen removed?

A

Splenectomy – causes infarction such as sickle cell anaemia, trauma, autoimmune haemolytic disease, infiltration (tumour), coeliac disease and congenital.

23
Q

Why does chemotherapy cause increased susceptibility to infections?

A
  • Chemotherapy causes neutropenia
  • Chemotherapy induces damage to mucosal barrier
  • Vascular catheters
24
Q

How should neutropenic septic patients be managed?

A

Treat suspected neutropenic sepsis as an acute medical emergency and offer empiric antibiotic therapy immediately. Assess patients risk of septic complications.

25
Q

What can cause SID due to increased loss or catabolism of immune components?

A

Protein losing conditions such as IgA nephropathy – deposition of IgA in the glomerulus and enteropathy.

26
Q

What is the importance of CRP?

A

CRP – acute phase protein that acts as an opsonin. It is triggered when endotoxins trigger the production of cytokines by monocytes and macrophages. These cytokines circulate in the blood to the liver where they stimulate the product of the acute phase proteins including CRP.

27
Q

What is Pus?

A

As neutrophils are drawn to the site of the infection they will phagocytose the bacteria. The neutrophils die as a result of bacterial toxins or from old age. The neutrophils are then broken down. An accumulation of dead neutrophils (and some other white cells) is called pus.